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Laryngeal lipoma associated with diffuse lipomatosis: case report and literature review.

Abstract

Lipomas are benign mesenchymal tumors that are often found in the head and neck. Intrinsic lipomas of the larynx and supraglottic area are rare, as fewer than 115 cases have been reported in the literature; almost all of these occurred in isolation. We report a case of a laryngeal lipoma that was associated with diffuse systemic lipomatosis. The tumor was successfully removed via an endoscopic laser resection. To the best of our knowledge, this is only the second case of laryngeal lipoma associated with lipomatosis to be reported in the English-language literature. We also review the literature on head and neck lipomas.

Introduction

Lipomas are slowly growing, benign mesenchymal tumors composed of adipose tissue. (1,2) They are usually solitary, encapsulated, subcutaneous or submucosal masses, and they primarily affect men (the male-to-female ratio is between 3:1 and 5:1). (2-4) Most cases occur in the sixth decade of life and later. (2-4)

Some 13% of all lipomas are found in the head and neck region, mostly in the posterior neck; lesions are rare in the anterior neck, infratemporal fossa, oral cavity, pharynx, larynx, and parotid gland. (3) Laryngeal lipomas account for only about 0.1% of all benign laryngeal tumors, but they should be considered in the differential diagnosis of a laryngeal mass. (2) These tumors are generally found in areas that contain a high proportion of subepithelial fat, such as the epiglottis and aryepiglottic folds. (4)

Patients most commonly present with slowly progressive airway obstruction and dysphagia. (5) Computed tomography (CT) and magnetic resonance imaging (MRI) can assist in the initial diagnosis of a laryngeal lipoma, but the final diagnosis must be made histologically. The treatment of choice is surgical excision via an endoscopic or open route.

Laryngeal lipomas are almost always solitary. We report a case of a large supraglottic lipoma that was associated with diffuse systemic lipomatosis. To the best of our knowledge, only 1 other such case has been reported in the English-language literature. (6)

Case report

A 57-year-old man with multiple subcutaneous lipomas presented with a 6-week history of progressive dysphagia with solids and a globus sensation. He reported no odynophagia, dyspnea, or change in voice, and he denied a history of relevant trauma or weight loss. He was a nonsmoker. He reported that when he had been in his 30s, he had undergone removal of multiple superficial lipomas.

On examination, numerous lipomas were present throughout his extremities and back (figure 1). Flexible laryngoscopy detected an obstructing mass arising from the tongue base with anterior displacement upon tongue protrusion. The true vocal folds were intact. CT demonstrated a 3.2 x 3.4 x 2.6-cm mass with fatty attenuation centered in the right aryepiglottic fold and right epiglottis (figure 2). A modified barium-swallow examination revealed a large, lucent mass extending over the midline and involving the right vallecula and epiglottis, although there was still adequate passage of the bolus around the mass (figure 3).

The patient was taken to the operating room for an endoscopic removal of the lesion. He was fiberoptically intubated and underwent suspension microlaryngoscopy, which demonstrated a well- circumscribed, encapsulated lesion located in the vallecula and encompassing the right aryepiglottic fold. The tongue-base mass was fully removed via a transoral endoscopic C[O.sub.2] laser resection; because the lesion was so large, the removal was performed in a piecemeal fashion (figure 4). The patient tolerated the procedure well, but because of the extent of the dissection and mucosal swelling, he remained intubated overnight. He was extubated on the first postoperative day without incident, and he resumed a regular diet. He was discharged home on postoperative day 2. The final pathologic analysis identified benign adipose tissue consistent with a lipoma.

At the 6-month follow-up examination, the larynx was well healed and the patient exhibited no evidence of disease (figure 5).

Discussion

The first case of laryngeal lipoma was reported in 1854 by Holt et al. (7) Since then, fewer than 115 cases have been reported in the English-language literature. (2,8)

Laryngeal lipomas may manifest with clinically adverse features. For example, in 2 previously reported cases, the presence of a large lipoma led to suffocation secondary to regurgitation into the hypopharynx. (9) Lipomas usually occur sporadically, but some are associated with several inherited disorders, including multiple lipomatosis, Gardner syndrome, and Madelung disease. (3) Recurrences, which have been reported in about 5% of patients, are more common when the lipoma is associated with generalized lipomatosis, when the mass is not completely removed at surgery, or when the tumor is a well-differentiated liposarcoma incorrectly diagnosed as a benign lipoma. (5,6) There are no reports of malignant transformation of a solitary lipoma, but it has occurred in cases of multiple laryngeal lipomas. (8)

[FIGURE 1 OMITTED]

[FIGURE 2 OMITTED]

Pathology. Laryngeal lipomas are composed of mature, white adipocytes. They are usually found in the supraglottic area because of the large amount of fat in this area. These masses are believed to develop from multipotential fibroblasts that differentiate into fat cells to form a lipoma. (1) The fat cells found in a lipoma are uniform in shape and size. Macroscopically, laryngeal lipomas are well-delineated, lightly colored, solitary masses that can be sessile or pedunculated and smooth or lobulated. (6)

[FIGURE 3 OMITTED]

Benign lipomas can be distinguished from well-differentiated liposarcomas because the former lack atypical lipocytes, lipoblasts, and cytologic evidence of pleomorphism, and because they are encapsulated and noninvasive. Only 30 cases of laryngeal liposarcomas have been reported in the literature, which means they are less prevalent than benign lipomas of the larynx. (10) Several subtypes of lipoma have been defined. They include fibrolipomas, which have a higher component of connective tissue; myxolipomas, which contain gelatinous stroma; spindle-cell lipomas, which feature fibroblastoid spindle cells; and angiolipomas, which are characterized by extensive vascularization. (2)

Diagnosis. The presenting signs and symptoms of laryngeal lipoma--airway obstruction, dysphagia, dyspnea, hoarseness, and globus pharyngeus--are the same as those of other benign and malignant tumors of the larynx) The most frequent complaint is airway obstruction. (5) Intervals of up to 25 years from onset to final diagnosis are not uncommon. Delays in diagnosis are attributable to the gradual development of nonspecific symptoms.

[FIGURE 4 OMITTED]

The differential diagnosis of a laryngeal mass includes hyperplastic mucosal polyp, mucus retention cyst, laryngocele, myoma, chondroma, papilloma, adenoma, lymphangioma, hemangioma, benign lipoma, and malignant liposarcoma. (2) The appearance of a well-differentiated liposarcoma can be very similar to that of a benign lipoma, so histopathologic analysis is imperative to establish the diagnosis.

Imaging. Both CT and MRI can demonstrate the extent of a tumor and establish its lipomatous nature, but neither can distinguish a lipoma from a liposarcoma. (8) Laryngeal lipomas have characteristic CT findings--namely, a noncontrast-enhancing homogeneous structure with few septations and low attenuation (Hounsfield units between -50 and -150) indicative of adipose tissue. (1) However, MRI is the preferred technique because it provides better definition of lipoma margins and better visualization of laryngeal musculature. (8) Lipoma intensity is highest on T1-weighted imaging.

Management. Treatment of laryngeal lipomas is surgical. Left untreated, lipomas can be life-threatening; cases of sudden airway obstruction have been reported. (1) Small tumors can be removed endoscopically with a cold-cutting electrosurgical or C[O.sub.2] laser. (2) In our patient, the tumor was quite large, so we performed a piecemeal excision to minimize surgical morbidity. For larger tumors, an external approach, such as a lateral or subhyoid pharyngotomy, may be required.

In order to prevent a recurrence, the surgical removal of a lipoma should be complete, and a histopathologic analysis should be performed to look for cellular atypia. (6) If the lesion is in fact a benign lipoma, complete excision is adequate. If the lesion is atypical, a complete excision with careful long-term follow-up is suggested because the recurrence rate in such cases is as high as 53%. (10) If the lesion is found to be a liposarcoma, postoperative radiotherapy should be considered. (8)

[FIGURE 5 OMITTED]

References

(1.) Talsania J, Shah VP, Shah AL, et al. Unusual case of laryngeal lipoma. Indian Journal of Otolaryngology and Head and Neck Surgery 2007; 59(1):85-6.

(2.) Lippert BM, Eggers S, Schluter E, et al. Lipoma of the larynx. Report of 2 cases and review of the literature. Otolaryngol Pol 2002;56 (6):669-74.

(3.) El-Monem MH, Gaafar AH, Magdy EA. Lipomas of the head and neck: Presentation variability and diagnostic work-up. J Laryngol Otol 2006;120(1):47-55.

(4.) Ortiz CL, Weber AL. Laryngeal lipoma. Ann Otol Rhinol Laryngol 1991;100(9 Pt 1):783-4.

(5.) Trizna Z, Forrai G, Toth B, Banhidy FG. Laryngeal lipoma. Ear Nose Throat J 1991;70(6):387-8.

(6.) Wenig BM. Lipomas of the larynx and hypopharynx: A review of the literature with the addition of three new cases. J Laryngol Otol 1995;109(4):353-7.

(7.) Holt B, Fergusson W, Partridge R. Fatty pendulous tumour of the pharynx and larynx. Trans Pathol Soc Lond 1854;5:123-5.

(8.) Singhal SK, Virk RS, Mohan H, et al. Myxolipoma of the epiglottis in an adult: A case report. Ear Nose Throat J 2005;84(11):728, 730, 734.

(9.) Jesberg N. Fibrolipoma of the pyriform sinuses: Thirty-seven year follow-up. Laryngoscope 1982;92(10 Pt 1): 1157-9.

(10.) Powitzky R, Powitzky ES, Garcia R. Liposarcoma of the larynx. Ann Otol Rhinol Laryngol 2007;116(6):418-24.

Megan L. Durr, MD; Nishant Agrawal, MD; John R. Saunders, MD; Patrick K. Ha, MD

From the Division of Head and Neck Cancer Research, Department of Otolaryngology--Head and Neck Surgery, Johns Hopkins Medical Institutions, Baltimore (Dr. Durr, Dr. Agrawal, and Dr. Ha), and Johns Hopkins Head and Neck Surgery at Greater Baltimore Medical Center, Milton J. Dance Jr. Head and Neck Center (Dr. Saunders and Dr. Ha).

Corresponding author: Patrick K. Ha, MD, Johns Hopkins Head and Neck Surgery at GBMC, 6569 N. Charles St., PPW Suite 401, Baltimore, MD 21204. E-mail: phal@jhmi.edu
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Title Annotation:ORIGINAL ARTICLE
Author:Durr, Megan L.; Agrawal, Nishant; Saunders, John R.; Ha, Patrick K.
Publication:Ear, Nose and Throat Journal
Article Type:Report
Geographic Code:1USA
Date:Jan 1, 2010
Words:1658
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